Abstract
Clinical features and treatment outcomes of primary cutaneous B-cell lymphoma (PCBCL) have rarely been reviewed, due to the rarity and pathologic obscurity of this disease. We reviewed 21 patients who were pathologically diagnosed with PCBCL from Samsung Medical Center's lymphoma cohort, following the WHO-EORTC classification system: primary cutaneous follicle-center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL, LT), and cutaneous diffuse large B-cell lymphoma, other (PCDLBCL, other). Of 2831 B-cell lymphoma cases, PCBCL comprised only 0.74 % of cases (N = 21, eight PCLBCL, LT (0.28 %), 10 PCMZL (0.35 %), two PCDLBCL, other (0.06 %), and one PCFCL (0.03 %)). Eighteen of 21 patients received treatment for PCBCL (12 chemotherapy alone, three radiotherapy alone, three chemotherapy following radiotherapy) and complete response (CR) was observed in 17 patients. The median progression-free survival was 44 months [95 % confidence interval (CI): 11-61 months]. Two patients had died at the time of analysis, with a median follow-up duration of 85 months [95 % confidence interval (CI): 55-118 months]. PCBCL cases in this study have a higher proportion of disseminated PCMZL and PCLBCL, LT, and excellent outcomes were observed with chemotherapy, including R-CHOP or R-CVP irrespective of staging and pathologic subtype.